Objectives are to understand canine globoid leukodystrophy (GLD) in relation to human GLD. In particular, we hope to explain GLD pathogenesis in morphologic terms and the nature and consequences of the enzyme defect responsible for the disease. Our current research goals are: 1) to continue maintenance of a colony of dogs for the production of GLD offspring; 2) to describe morphologic events in the pathogenesis of GLD neuropathy at the ultrastructural level; 3) to quantify the distribution of lesions in canine GLD and discover regional features correlated with susceptibility vs. resistance to GLD lesions; 4) to collaborate with Dr. Kunihiko Suzuki in evaluating enzyme activity in GLD and normal control dogs.